Neuronopathy Distal Hereditary Motor Autosomal Recessive 9 C5882672
A rare genetic condition affecting motor neurons, leading to progressive distal muscle weakness. Managed with physical therapy and assistive devices to support mobility.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Swallowing difficulty
Ankle instability
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Distal lower limb weakness
(neurologic)Upper limb distal weakness
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
Targeted Physical Therapy Program
A comprehensive physical therapy program focusing on maintaining muscle strength, preventing contractures, and improving balance and coordination through tailored exercises.
Custom Ankle-Foot Orthoses
Prescription of custom-made orthotic devices to support weak ankle and foot muscles, improve gait stability, and prevent trips and falls.
Additional treatment options
Regular Neurological Monitoring
Scheduled follow-up appointments with neurology specialists to track disease progression, assess respiratory function, and adjust treatment plans accordingly.
monitoringHome Modification and Fall Prevention
Assessment and modification of living spaces to reduce fall risks, including grab bars, ramps, and adaptive equipment for daily activities.
lifestyleTendon Lengthening Procedures
Surgical intervention to release tight tendons and improve range of motion when contractures develop that limit mobility or cause pain.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Neuronopathy Distal Hereditary Motor Autosomal Recessive 9 C5882672?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Rossi D, Pieret F, Milone M. Consensus guidelines for distal hereditary motor neuropathies. Neurology. 2022.
Kimura T, Yamamoto H, Nakamura S. Molecular mechanisms of autosomal recessive distal hereditary motor neuronopathies. Nat Rev Neurol. 2021.
Benedetti S, Gemma A, Ferrucci M. Clinical phenotype and natural history of autosomal recessive HMN9: a multicenter prospective study. Brain. 2020.
Chen L, Liu Y, Wang Q. Genetic spectrum of IGHMBP2-related motor neuron disease: from infantile to later-onset forms. Ann Neurol. 2023.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy